Idiopathic pulmonary fibrosis (IPF) is a lung disease in which lung tissue becomes progressively more scarred and stiff. This makes it more and more difficult to breathe.

There’s currently no cure for IPF, but new drugs have been able to slow the rate of decline and improve quality of life. Other treatment possibilities include supplemental oxygen, pulmonary rehabilitation to help you breathe better, and lung transplants. Experimental research studies are ongoing to find new treatments.

Early IPF treatment is important because it can slow the progression of the disease and improve your quality of life. It also contributes to knowledge of IPF and the results of different treatment courses on life expectancy. Treatments include:

  • Medication: New drug treatments may slow the rate of IPF lung scarring. This is important, because lung scarring is irreversible.
  • Supplemental oxygen and physical therapy: These improve lung function, which can help you manage IPF and function more normally.
  • Exercise: Maintaining and increasing your muscle mass can improve your survival time, according to a recent study.
  • A lung transplant: This may significantly prolong your life. The younger you are, the better qualified you’ll be for a transplant.
  • GERD treatment: Taking medication for gastroesophageal reflux disease (GERD), which most people with IPF have, is associated with less lung scarring and longer survival time.

Your doctor will discuss with you what treatment regimen may be best for your particular case.

New drugs

The most important development in treating IPF is the availability of new drugs. In 2014, the U.S. Food and Drug Administration (FDA) approved the use of two new drugs for IPF: nintedanib (Ofev) and pirfenidone (Esbriet). The drugs don’t cure IPF, but they help to prevent further scarring and slow the progression of the disease. Medical studies report that both drugs produced “a statistically significant slowing” in the decline of lung function. The same studies indicated that nintedanib produced somewhat better results than pirfenidone.

Supportive treatments

The standard care for IPF is supportive. A small portable oxygen tank can supply additional oxygen to help you breathe, especially when you’re more active. This is important for your comfort and also to prevent right-sided heart problems caused by low oxygen levels in your blood.

Pulmonary rehabilitation is a program designed to help you cope with IPF and improve your quality of life. It involves exercises in breathing, stress reduction, and education. One recent study showed that exercise training improved lung function.

Lung transplant

Lung transplantation can improve your quality of life and your lifespan, but it also has some risks. IPF is now a leading reason for lung transplants in the United States, accounting for almost half of the lung transplants that were performed in 2013.

Besides medical treatment options, there are things you can do to help manage and live better with the disease:

  • If you smoke, stop. Smoking is correlated with the occurrence of IPF, and smoking aggravates the disease.
  • Lose weight if you are overweight, and maintain a healthy weight. Extra weight makes it harder to breathe.
  • Keep up to date with flu and pneumonia vaccinations. Both illnesses are harmful to people with IPF.
  • Treat gastroesophageal reflux or sleep apnea if you have them. These are often present in IPF patients.
  • Monitor your oxygen levels at home.
  • Take vitamin and mineral supplements, as recommended.
  • Join an IPF support group.